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Objective: Behçet's syndrome (BS) is a multisystem variable vessel vasculitis characterized by skinmucosal lesions. It can also involve the eyes, blood vessels, joints, gastrointestinal system, urogenital system, and central nervous system. BS starts in the third or fourth decade and affects both genders equally. The disease is more severe in young men. Although the sensitivity of the pathergy test (PT) is decreasing today, it is still an important clue in the diagnosis of BS. We describe the characteristics of BS in our region, retrospectively. We also analyzed the effect of gender, age, family history, and skin PT positivity status on the difference of clinical involvement. Methods A total of 777 BS patients (391 women and 386 men; 40.0 ± 11.6 years old) who applied to our Rheumatology Department between January 2010 and June 2020 were included in the study. Results: Of the 777 patients, 391 were female (50.3%) and 386 were male (49.7%). The mean age at diagnosis was 30.3 ± 9.8 years. The proportion of patients with BS in their family was 10.2%. Of the 777 patients, 310 (39.9%) had only mucocutaneous symptoms. Other 467 patients (60.1%) had at least one of the ocular, musculoskeletal, vascular, neurologic, intestinal, or genitourinary involvement. Serious involvements such as eye, cardiovascular, and neurologic involvement were more common in male patients. Conclusion: BS has the different clinical phenotypes according to gender, age of onset, and skin PT positivity status. Gender influences on the major organ involvements such as eye, neurologic, and vascular. [ABSTRACT FROM AUTHOR]

Objective: Behçet's disease is heterogeneous with clinical variability across ethnicities and geographic locations. The goal of this study was to analyze the clinical characteristics of our multi-ethnic Behçet's disease cohort at the University of Michigan. Material and Methods: A detailed patient characterization was performed. Differences in disease characteristics between men and women, and between patients fulfilling the International Criteria for Behçet's Disease (ICBD) and the International Study Group criteria (ISG) were determined in our cohort. Results: A total of 114 patients with a male to female ratio of ~ 1:4 were included. All patients met the ICBD criteria, including 76 who also met the ISG criteria. Over 95% of patients had recurrent genital ulcers, which is higher than generally reported. Retinitis was 5.3 times more likely in men than in women (p=0.009), and arthralgia was 3.3 times more likely in women than men (p=0.048). When comparing cohorts derived from the two different criteria, the ISG cohort had more skin manifestations (OR=3.3, p=0.0006). Acneiform lesions were associated with ~8 times higher odds of developing retinitis in our patients (p=0.0008), and superficial thrombophlebitis was associated with a trend for higher odds of developing uveitis (OR=4.1, p=0.057). Using the ICBD criteria, 38 additional patients were identified compared to only using the ISG criteria. Of these patients, 28 presented with only mucosal ulceration with or without joint involvement. Conclusion: We characterize Behçet's disease in a multi-ethnic cohort from North America. Using ICBD criteria in the United States significantly increases the likelihood of identifying Behçet's disease, particularly in patients with isolated mucosal involvement who constitute a substantial subset of patients in this region. [ABSTRACT FROM AUTHOR]